BACKGROUND: Paraesophageal hernias are quite common and sometimes feared due to the risk of incarceration and strangulation of any herniated organ. The hereby reported combination of an incarcerated paraesophageal hernia containing a perforated peptic ulcer is extremely rare. CASE PRESENTATION: An elderly man with multiple medical conditions was admitted due to severe upper abdominal pain. The patient was found to have a paraesophageal hernia and underwent a laparotomy. In the hernia, a perforated benign peptic duodenal ulcer was found. The duodenal defect was over-sewn, the hernial defect was closed and the former hernial cavity was drained by a right-sided chest tube. The patient was discharged one month after surgery and was found to do well at follow-up one month after discharge. CONCLUSION: This is the first report of a patient surviving the extremely rare and life-threatening combination of a perforated peptic duodenal ulcer in a paraesophageal hernia.